Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Mar 17;23(1):60.
doi: 10.1186/s12931-022-01982-2.

A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP)

Francesca Mariani  1 , Elena Salvaterra  2   3 , Sara Lettieri  2 , Annalisa De Silvestri  4 , Alessandra Corino  1 , Matteo Bosio  1 , Elia Fraolini  1 , Davide Piloni  5   6 , Giuseppe Rodi  7 , Angelo Guido Corsico  1   2 , Ilaria Campo  1
Affiliations

A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP)

Francesca Mariani et al. Respir Res. .

Abstract

Background: PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still debated. Considering ICU hospitalization associated risks, a "mini-WLL" with anticipated manual clapping and reduced total infusion volume and has been proposed in our center. The aim of the study is to retrospectively analyze the efficacy of mini-WLL compared to standard WLL at the Pavia center.

Methods: 13 autoimmune PAP patients eligible for WLL were included: 7 patients were admitted to mini-WLL (9 L total infusion volume for each lung) and 6 patients underwent standard WLL (14 L of infusion volume). Functional data (VC%, FVC%, TLC%, DLCO%) and alveolar-arterial gradient values (A-aO2) were collected at the baseline and 1, 3, 6, 12, 18 months after the procedure.

Results: A statistically significant improvement of VC% (p = 0.013, 95%CI 3.49-30.19), FVC% (p = 0.016, 95%CI 3.37-32.09), TLC% (p = 0.001, 95%CI 7.38-30.34) was observed in the mini-WLL group in comparison with the standard WLL group, while no significant difference in DLCO% and A-aO2 mean values were reported.

Conclusion: Mini-WLL has demonstrated higher efficacy in ameliorating lung volumes, suggesting that a lower infusion volume is sufficient to remove the surfactant accumulation and possibly allows a reduced mechanical insult of the bronchi walls and the alveoli. However, no statistically significant differences were found in terms of DLCO% and Aa-O2.

Keywords: ICU stay; Pulmonary alveolar proteinosis; Whole lung lavage.

PubMed Disclaimer

Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Pulmonary function test performed 1 month before the WLL and 3, 6, 12, 18 months after the treatment. a VC%, b FVC% and c TLC% of predicted volumes. Continuous line = mini-WLL; dotted line = standard WLL. Vertical bars represent confidence interval
See this image and copyright information in PMC

References

    1. Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002;166:215–235n. doi: 10.1164/rccm.2109105. - DOI - PubMed
    1. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med. 2003;349:2527–2539. doi: 10.1056/NEJMra023226. - DOI - PubMed
    1. Salvaterra E, Campo I. Pulmonary alveolar proteinosis: from classification to therapy. Breathe (Sheff). 2020;16(2):200018. - PMC - PubMed
    1. Kitamura T, Tanaka N, Watanabe J, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte macrophage colony stimulating factor. J Exp Med. 1999;190(875–80):81. - PMC - PubMed
    1. Uchida K, Nakata K, Suzuki T, et al. Granulocyte/macrophage colony-stimulating factor autoantibodies and myeloid cell in healthy individuals. Blood. 2009;113:2547–2556. doi: 10.1182/blood-2008-05-155689. - DOI - PMC - PubMed

Substances

Supplementary concepts